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Literature summary for 3.2.1.46 extracted from

  • Zhang, X.Y.; Dinh, A.; Cronin, J.; Li, S.C.; Reiser, J.
    Cellular uptake and lysosomal delivery of galactocerebrosidase tagged with the HIV Tat protein transduction domain (2008), J. Neurochem., 104, 1055-1064.
    View publication on PubMed

Application

Application Comment Organism
medicine findings could lead to an improved therapy for globoid cell leukodystrophy, GLD, or Krabbe disease Mus musculus

Cloned(Commentary)

Cloned (Comment) Organism
expression vectors for different fusion-proteins are constructed: GALC, unmodified enzyme, GALC-MH: fusion protein containing a C-terminal myc-tag and six His residues, GALC-TMH: fusion protein containing in addition a Tat-PTD, the protein transduction domain derived from the HIV-1 Tat protein Mus musculus

Protein Variants

Protein Variants Comment Organism
GALCdelta-MH mutant containing a C-terminal myc-tag and six His residues with the last 11 amino acids at the C-terminus of GALC deleted Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Mus musculus 5764
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
80000
-
determined by SDS-PAGE and Western Blot analysis Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
D-galactosyl-N-acylsphingosine + H2O Mus musculus
-
D-galactose + N-acylsphingosine
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mus musculus
-
myelin sheath
-
Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
distribution of recombinant GALC activity in COS-7 cells: GALC-MH, 41.2% activity in cell extract, 58.8% released into cell culture medium, GALC-TMH, 27.5% to 72.5% Mus musculus
70
-
with plasmid encoding GALC-TMH transfected 293T cell, GALC units per mg of cell extract Mus musculus
120
-
with plasmid encoding GALC-MH transfected 293T cell, GALC units per mg of cell extract Mus musculus
130
-
with plasmid encoding unmodified GALC transfected 293T cell, GALC units per mg of cell extract Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
D-galactosyl-N-acylsphingosine + H2O
-
Mus musculus D-galactose + N-acylsphingosine
-
?
psychosine + H2O galactosylsphingosine Mus musculus D-galactose + sphingosine
-
?

Synonyms

Synonyms Comment Organism
galactocerebrosidase
-
Mus musculus
Galactosylceramidase
-
Mus musculus
GALC
-
Mus musculus

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
4.2
-
-
Mus musculus